Contents
Incidence
Non Hodgkin's lymphoma was the tenth most commonly diagnosed malignancy in Australia in 2010.[1] Hodgkin lymphoma (HL) comprises approximately 10% of lymphomas and 0.6% cancers worldwide.
Classification of haematopoietic and lymphoid malignancies
Various classifications have been used in older literature sources, however in current usage is the World Health Organization (WHO) classification of 2001, updated in 2008. This incorporated the new knowledge of both immunophenotypic and cytogenetic information.[2] The focus is on lineage derivation into myeloid, lymphoid or dendritic/histiocytic entities, though there is sometimes biphenotypic differentiation or “lineage infidelity”, suggesting derivation from multipotent progenitor cells.[3]
Myeloid: |
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AML is inevitably aggressive in its course and needs immediate therapy. It may be a primary or secondary neoplastic change, consequent on a prior immunological or haematological disturbance or therapy (eg prior chemotherapy or radiation).
There may be an overlap between MPN and MDS and the course is very variable, but may evolve to AML.
Lymphoid: |
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Histiocytic/dendritc neoplasms: |
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Background history
Connective tissue disorders as precursors for a secondary LPD. Also some increased risk in family members for HL. Environmental exposures and past chemotherapy or radiotherapy as risk factors for a secondary leukaemia.
Common presentations
Leukaemia
Cytopenia: |
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Lymphoma
Risk factors include HIV/EBV/ HCV infection, family history of lymphoprolferative disorder (LPD), background autoimmune disorders for secondary lymphoproliferative disorder, inflammatory gastrointestinal disease (e.g. Crohn’s disease, coeliac disease, Helicobacter pylori chronic gastritis), environmental exposures (see Table 2).[5]
Mass disease: |
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Constitutional disturbance: |
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Metabolic disturbance: |
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Immune phenomena: |
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Paraneoplastic phenomena: |
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Myeloma
Cytopenia: |
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Mass disease: |
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Metabolic disturbance: |
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Coincidental finding: |
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Definitive diagnosis
Biopsy
Fine needle aspiration cytology may help define broad lymph node pathology, but architectural features and more cellular material obtained by an excision biopsy allows full classification. Cytogenetics, flow cytometry and gene rearrangements aid subclassification.
Bone marrow examination is routine and CSF may be sampled, especially in aggressive lymphoma histologies such as Burkitt’s lymphoma and leukaemia/lymphoma, where it may be a “sanctuary site” for later relapse, as may be the testes.
Bone marrow aspiration and trephine biopsy are essential to definitively diagnose myeloma and differentiate it from monoclonal gammopathy of undetermined significance (MGUS).
Staging
Staging the extent of both NHL and HL is based on Ann Arbor classification Table 3, Ref. 4) as determined by physical findings, CT, MRI and PET scan findings (see LN location Figure 1).[5] In addition, risk stratification is assisted by use of the Revised International Prognostic Index (R-IPI) and follicular lymphoma prognostic index (FLIPI) (see Table 3[6] and Table 4[7]).
Myeloma assessment is by skeletal survey to determine the volume of lytic disease in marrow bearing bones, checking for end-organ damage in the form of hypercalcaemia/anaemia/renal failure, determining the burden of disease related to paraprotein concentration (Durie and Salmon system 1975 and Table 5).[8] Serum and urine (Bence Jones) electrophoresis and immunofixation
Management: |
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Prognosis: |
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References
- ↑ Australian Institute of Health and Welfare. Cancer in Australia: key facts. [homepage on the internet] Canberra: AIHW; 2013 [cited 2014 May 22; updated 2013]. Available from: http://www.aihw.gov.au/cancer/cancer-in-australia/.
- ↑ Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H. WHO classification of tumours of haematopoietic and lymphoid tissues, fourth edition. Lyon, France: IARC; 2008 [cited 2014 May 22] Available from: http://apps.who.int/bookorders/anglais/detart1.jsp?sesslan=1&codlan=1&codcol=70&codcch=4002.
- ↑ Freedman AS, Friedberg JW, Aster JC. Classification of the hematopoietic neoplasms. [serial online]: UpToDate; 2013 Sep 9 [cited 2014 May 22] Available from: http://www.uptodate.com/contents/classification-of-the-hematopoietic-neoplasms?source=search_result&search=Classification+of+the+hematopoietic+neoplasms&selectedTitle=1~150.
- ↑ Aster JC. Epidemiology, pathologic features, and diagnosis of classical Hodgkin lymphoma. [serial online]: UpToDate; 2014 Jan 14 [cited 2014 May 22] Available from: http://www.uptodate.com/contents/epidemiology-pathologic-features-and-diagnosis-of-classical-hodgkin-lymphoma.
- ↑ 5.0 5.1 Freedman AS, Friedberg JW, Aster JC. Clinical presentation and diagnosis of non-Hodgkin lymphoma. [serial online]: UpToDate; 2014 Feb 24 [cited 2014 May 22] Available from: http://www.uptodate.com/contents/clinical-presentation-and-diagnosis-of-non-hodgkin-lymphoma?source=search_result&search=Clinical+presentation+and+diagnosis+of+non-Hodgkin+lymphoma&selectedTitle=1~150.
- ↑ Sehn LH, Berry B, Chhanabhai M, Fitzgerald C, Gill K, Hoskins P, et al. The revised International Prognostic Index (R-IPI) is a better predictor of outcome than the standard IPI for patients with diffuse large B-cell lymphoma treated with R-CHOP. Blood 2007 Mar 1;109(5):1857-61 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/17105812.
- ↑ Solal-Céligny P, Roy P, Colombat P, White J, Armitage JO, Arranz-Saez R, et al. Follicular lymphoma international prognostic index. Blood 2004 Sep 1;104(5):1258-65 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/15126323.
- ↑ Rajkumar SV. Clinical features, laboratory manifestations, and diagnosis of multiple myeloma. [serial online]: UpToDate; 2014 Apr 4 [cited 2014 May 22] Available from: http://www.uptodate.com/contents/clinical-features-laboratory-manifestations-and-diagnosis-of-multiple-myeloma?source=search_result&search=Rajkumar+SV.+Clinical+features%2C+laboratory+manifestations%2C+and+diagnosis+of+multiple+myeloma&selectedTitle=2~150.
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